Clotting Disorders Flashcards
What is the management of immune thrombocytopaenic purpura?
Oral prednisolone
IVIg and Ritixumab 2nd line
Splenectomy rarely
What is the management of immune thrombocytopaenic purpura in children?
None - self-resolves in 6 months
Steroids and IVIg if platelets <10
Platelet transfusion if major bleed
How does immune thrombocytopenic purpura present?
Mild bleeding - bruising, petichiae, gums, epistaxis
Rarely presents with significant bleeding (eg. intercranial)
What is the pathophysiology thrombotic thrombocytopaenic purpura?
What is the mutation?
ADAMSTS13 deficiency causes vWF build up in small vessels
How does thrombotic thrombocytopaenic purpura present?
Hint: the terrible pentad
Haemolytic anaemia Thrombocytopaenia Fever Neurological signs - from micro emboli Renal dysfunction
What blood film finding may be seen in thrombotic thrombocytopaenic purpura?
Schistocytes
What is the management for thrombotic thrombocytopaenic purpura?
Plasma exchange
Steroids and Ritixumab 2nd line
How is heparin-induced thrombocytopaenic purpura treated?
Switching from heparin to alternative anticoagulant
What 3 features may be found in a blood test of haemolytic uraemic syndrome?
Haemolytic anaemia
Thrombocytopaenia
AKI picture (raised urea/creatinine)
Schistocytes on blood film
What d-dimer, aPPT and fibrinogen results would you expect for DIC?
Raised d-dimer
Raised aPPT
Low fibrinogen
How is type 1 and type 2 of Von Willebrand disease inherited?
Type 1 - autosomal dominant
Type 2 - autosomal recessive
What clotting factor is low in Von Willebrand disease?
Factor VIII
When would you treat Von Willebrand disease?
What is the management?
Don’t treat regularly, only in the context of surgery or trauma.
Desmopressin - raises vWF
vWF infusion
Factor VIII concentrate
What is the management for menorrhagia in Von Willebrand disease?
Tranexamic acid Mefanamic acid Norithestone COCP Mirena coil Hysterectomy
What factor is absent in Haemophilia A?
Factor VIII
